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Current Perspective on Retinal Migraine

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Current Perspective on Retinal Migraine vision Review Current Perspective on Retinal Migraine Yu Jeat Chong 1, Susan P. Mollan 1 , Abison Logeswaran 2, Alexandra B. Sinclair 3,4 and Benjamin R. Wakerley 3,4,* 1 Birmingham Neuro-Ophthalmology, Ophthalmology Department, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2TH, UK; [email protected] (Y.J.C.); [email protected] (S.P.M.) 2 Moorfields Eye Hospital NHS Foundation Trust, London EC1V 2PD, UK; [email protected] 3 Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2TH, UK; [email protected] 4 Metabolic Neurology Group, University of Birmingham, Edgbaston, Birmingham B15 2TT, UK * Correspondence: [email protected] Abstract: Retinal migraine was first formally described in 1882. Various terms such as “ocular migraine” and “ophthalmic migraine” have since been used interchangeably in the literature. The lack of a consistent consensus-based definition has led to controversy and potential confusion for clinicians and patients. Retinal migraine as defined by the International Classification of Headache Disorders (ICHD) has been found to be rare. The latest ICHD defined retinal migraine as ‘repeated attacks of monocular visual disturbance, including scintillation, scotoma or blindness, associated with migraine headache’, which are fully reversible. Retinal migraine should be considered a diagnosis of exclusion, which requires other causes of transient monocular visual loss to be excluded. The aim of this narrative review is to summarize the literature on retinal migraine, including: epidemiology and risk factors; proposed aetiology; clinical presentation; and management strategies. It is potentially a misnomer as its proposed aetiology is different from our current understanding of the mechanism of migraine Keywords: retinal migraine; ocular migraine; ophthalmic migraine; migraine aura Citation: Chong, Y.J.; Mollan, S.P.; Logeswaran, A.; Sinclair, A.B.; Wakerley, B.R. Current Perspective on Retinal Migraine. Vision 2021, 5, 38. 1. Introduction and Historical Evolution https://doi.org/10.3390/vision There has been extensive documentation of migraine as a disorder, dating back to over 5030038 4000 years ago from ancient Mesopotamia [1]. One of the earliest descriptions of visual Received: 28 June 2021 symptoms associated with migraine was a case report by Hippocrates (c. 460—c. 370 BC) in Accepted: 18 August 2021 ancient Greece [1]: Published: 23 August 2021 ‘he seemed to see something shining before him like a light, usually in part of the right eye; at the end of a moment, a violent pain supervened in the right temple, Publisher’s Note: MDPI stays neutral then in all the head and neck. vomiting, when it became possible, was able to with regard to jurisdictional claims in divert the pain and render it more moderate.’ published maps and institutional affil- iations. In 1882, Galezowski first used the term ophthalmic megrim to describe a case series of four patients with permanent monocular visual loss and migraine headaches. He hypothesized that ophthalmic megrim is a condition that may ‘occasionally lead to organic changes in the retina or retinal vessels’, with findings such as retinal thrombosis and optic disc atrophy [2]. Copyright: © 2021 by the authors. The term retinal migraine was first introduced by Caroll in 1970 when he suggested Licensee MDPI, Basel, Switzerland. that although controversial, it could ‘possibly be applied to uncommon cases’ of migraine [3]. This article is an open access article distributed under the terms and He presented a case series of 15 patients with monocular visual loss without associated conditions of the Creative Commons features of headache, mostly transient with a duration of less than 10 min and rarely lasting Attribution (CC BY) license (https:// more than an hour. A few cases had permanent visual loss resulting in optic atrophy, creativecommons.org/licenses/by/ which he hypothesized was secondary to raised intraocular pressure from paroxysmal 4.0/). vasoconstriction of the central retinal artery or the ophthalmic artery. Vision 2021, 5, 38. https://doi.org/10.3390/vision5030038 https://www.mdpi.com/journal/vision Vision 2021, 5, 38 2 of 7 Without an internationally agreed definition, authors have used various terms inter- changeably to describe monocular visual loss, which is either: associated with headache, not associated with headache, transient, or permanent. These terms include ‘ophthalmic migraine’, ‘ocular migraine’, and anterior pathway migraine [4–7]. Retinal migraine was first included in the 1988 edition of the International Classifi- cation of Headache Disorders (ICHD) with an internationally agreed definition [8]. In the latest 2018 ICHD-3, retinal migraine is defined as ‘repeated attacks of monocular vi- sual disturbance, including scintillation, scotoma, or blindness, associated with migraine headache’ [9]. These episodes are fully reversible. The full diagnostic criteria of retinal migraine are included in Box1. In this narrative review, we have summarized the literature on retinal migraine as defined by the latest ICHD, to include epidemiology and risk factors, proposed aetiology, clinical presentation, and management strategies. Box 1. ICHD classification of retinal migraine [9]. 1. Attacks fulfilling criteria for migraine with aura and the criterion below 2. Attacks fulfilling criteria for migraine without aura and the criterion below 3. Aura characterized by both of the following: (A) Fully reversible, monocular, positive, and/or negative visual phenomena (e.g., scintillations, scotomata or blindness) confirmed during an attack by either or both of the following: • clinical visual field examination • the patient’s drawing of a monocular field defect (made after clear instruction) (B) At least two of the following: • spreading gradually over ≥5 min • symptoms last 5–60 min • accompanied, or followed within 60 min, by headache 4. Other causes of amaurosis fugax were excluded, and an episode cannot be accounted for by another ICHD-3 diagnosis. This is a diagnosis of exclusion. The ICHD-3 criteria has to meet points 1, 3(A), 3(B), and 4; while the ICHD-2 criteria has to meet points 2, 3(A), and 4 for a diagnosis of retinal migraine to be made. In the ICHD-2, it was specified that there should be normal ophthalmological examinations between attacks. 2. Methods A narrative review was conducted based on the Scale for the Assessment of Narrative Review Articles (SANRA) principles due to its broad scope and ability to include a range of information sources. The following databases were searched by the authors Y.Y.C. and A.L.: Embase Classic, Embase, and Ovid MEDLINE. We used the search term ‘retinal migraine’ and identified 270 articles. All studies included had a clear research question, appropriate study design, and discussion of results. Non-English articles and conference proceedings were excluded. Additional articles that were relevant were hand-searched from Google Scholar and also identified from citations within articles. 3. Epidemiology and Risk Factors Retinal migraine is a rare condition and its true incidence is difficult to ascertain due to a number of factors. Firstly, a consensus-based diagnostic criteria was only established in the 1988 ICHD. The 1988 ICHD defined retinal migraine as ‘at least two attacks’ of fully reversible monocular visual disturbance with typical migraine headache, normal neuro-ophthalmic examination, and exclusion of other causes [8]. Secondly, the diagnostic criteria of retinal migraine has continued to evolve with time. In 2004, the revised ICHD-2 maintained the same diagnostic criteria, with the difference that it is associated with headaches that fulfill the criteria for migraine without aura [10]. Grosberg et al., in 2006, summarized the clinical features and prognosis of the largest case series of retinal migraine patients (40 cases from previous literature, with 6 new cases) based on the ICHD-2 criteria [11]. Contrary to the ICHD-2 criteria of migraine without Vision 2021, 5, 38 3 of 7 aura, they showed that half of the patients meeting the definition of retinal migraine had a history of migraine with aura. The 2018 ICHD-3 criteria was subsequently updated to ‘attacks fulfilling criteria for migraine with aura’ [9]. The same authors also showed that out of the 46 patients with retinal migraine, 28 (61%) were female, with the age of onset ranging from 7 to 54 years old [11]. They further subcategorized patients based on whether they only showed transient visual loss (TVL), or subsequently developed permanent visual loss (PVL). Twenty one out of 46 (46%) patients experienced PVL, with a preponderance for females (15 females compared to 6 males). The mean age of onset was similar overall in both groups, at 24.7 years for the TVL group and 23.0 years for the PVL group. In females, the peak age of onset was the third and fourth decade for the TVL group, and second and third decade for the PVL group. A family history of migraine was documented in 14 out of 46 (30%) of patients. Thirdly, there are discrepancies in the literature with regards to inclusion criteria for retinal migraine. Another literature review based on the ICHD-2 criteria was conducted by Hill et al. in 2007, which included patients from Grosberg et al. in 2006 [12]. Of the 142 patients identified, 103 had transient monocular vision loss (TMVL) attributed to retinal migraine. Of the 103, only 16 had clinical manifestations for definite retinal migraine with at least two episodes of TMVL and headaches with a migraine-like phenotype. Of these 16, only five met the full ICHD-2 criteria for retinal migraine. Six out of 16 were female, with a mean age of 25.5 years (ranging from 9 to 53 years). Discrepancies between Grosberg et al. in 2006 and Hill et al. in 2007 are based on several points of contention. Grosberg et al. included patients who had a single episode of fully reversible monocular visual phenomena occurring during or within 60 min of a migraine attack; while Hill et al.
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